UNASSIGNED: Venetoclax is a BCL-2 inhibitor with proven efficacy in patients with multiple myeloma (MM) and translocation t(11;14). However, its role in plasma cell leukemia (PCL) remains unclear. Herein, we aimed to report a case of relapsed MM with secondary PCL and t(11;14) achieving complete (CR) and durable remission with venetoclax therapy.
UNASSIGNED: A 52-year-old gentleman was diagnosed with MM-free light chain lambda (ISS III) in December 2016. He received induction therapy, followed by autologous stem cell transplant. (ASCT) in May 2017 and maintenance. A year later, the patient relapsed with secondary PCL. His cytogenetics analysis revealed t(11; 14). The patient failed salvage chemotherapy and was shifted to venetoclax with dexamethasone treatment. The patient attained complete remission (CR), which was maintained for two years and a half before he developed fatal COVID-19 pneumonia.
UNASSIGNED: In comparison with the reported literature, this case report offers the latest compilation of the available evidence on the use of venetoclax in patients with PCL. Furthermore, our patient achieved CR for the longest reported durable response in literature thus far. Prospective clinical trials are needed to elucidate the optimal dosage, combination, and duration of treatment, ensuring better representation and generalizability of the findings. Meanwhile, venetoclax may be considered as a therapeutic option in patients with PCL t(11;14).

Background: T-prolymphocytic leukemia (T-PLL) is an aggressive hematologic malignancy. A portion of patients can be cured with alemtuzumab induction followed by allogeneic hematopoietic stem cell transplant, but patients who relapse after transplant have a poor prognosis, and there is no standard of care.Methods: We report a case of a 64-year-old man with relapsed JAK3-mutant T-PLL following allogeneic transplant who was treated with ruxolitinib and venetoclax.Results: Treatment with ruxolitinib and venetoclax resulted in a partial response including stabilization of the peripheral lymphocyte count, improvement in thrombocytopenia, decrease in splenomegaly, and a numerical reduction in the percentage of bone marrow involved by T-PLL. The combination was well tolerated with the exception of neutropenic infections.Conclusion: This case adds to the growing body of literature supporting venetoclax and rituximab as a viable treatment option for relapsed/refractory T-PLL with JAK-STAT alterations.

UNASSIGNED: This study aimed to improve the understanding of acute myeloid leukemia (AML) secondary to chronic lymphocytic leukemia (CLL), and to explore the sequence of occurrence and clonal origin of the two diseases.
UNASSIGNED: We reported a case of a 71-year-old man with a history of CLL. The patient was administrated with chlorambucil for 19 years and was admitted to our hospital due to fever. Then he was subjected with routine blood tests, bone marrow smear examination, flow cytometric immunophenotyping and cytogenetic analysis. A final diagnosis of AML-M2 secondary to CLL with -Y,del(4q),del(5q),-7,add(12p),der(17),der(18),-22,+mar was made. After rejecting the therapy with Azacitidine combined with B-cell lymphoma-2 (Bcl-2) inhibitor, the patient died of pulmonary infection.
UNASSIGNED: This case highlights the rare occurrence of AML secondary to CLL after prolonged chlorambucil therapy and the poor prognosis of such cases, underscoring the importance of enhanced assessment of these patients.

Lymphoma is the most common orbital malignancy in adults. Among the types of lymphoma, mantle cell lymphoma is a particularly aggressive form, often discovered through systemic involvement, with a dismal prognosis due to frequent recurrences. It is secondary to a t (11 ; 14) (q13; q32) chromosomal translocation resulting in an anti-apoptotic signal via overexpression of Bcl-2. Treatment is based on R-CHOP poly-chemotherapy. We describe the case of a patient with an orbital recurrence of mantle cell lymphoma successfully treated with oral Bcl-2 inhibitor monotherapy. A 58-year-old man who was treated with R-CHOP 8 years ago for mantle cell lymphoma, in remission for 5 years, presented with progressive decreased visual acuity in the left eye, along with binocular diplopia. Clinical examination revealed a decrease in visual acuity in the left eye to 1/20 Parinaud 20 and a relative afferent pupillary defect on the left. External examination revealed a left cranial nerve VI palsy, 2mm of painless proptosis, and hypesthesia of the left V1 territory, leading to a diagnosis of left orbital apex syndrome. The disc and macular OCT were normal. The visual field showed enlargement of the left blind spot. An emergency CT scan and MRI revealed an apical extraconal tissue mass infiltrating the medial rectus muscle, extending to the superior orbital fissure, optic canal and left cavernous sinus, hyperintense on T2 weighted images and isointense on T1. The morphological appearance was strongly suggestive of an infiltrative lymphomatous process. An 18 FDG PET-scan identified the orbital lesion as well as enhancing lesions in the axilla and colon; given the clinical features and test results, the diagnosis of recurrent mantle cell lymphoma was made without biopsy. Treatment with Venetoclax (Bcl-2 inhibitor) was initiated. At one month of treatment, the orbital apex syndrome had entirely resolved, with visual acuity increased to 8/10 Parinaud 4 and a metabolic return to normal on PET scan. The PET scanner and clinical examination at 3 months were entirely normal. At the one-year follow-up visit, the patient was still on Venetoclax, the clinical examination was unchanged, and the PET-scan still showed a complete metabolic response.

Objective: To improve the knowledge and experience of ibrutinib combined with CAR-T cells in the treatment of high-risk chronic lymphoblastic leukemia (CLL) patients or small lymphocytic lymphoma (SLL) with TP53 gene aberration. Methods: One case of del (17p) CLL patients with BCL-2 inhibitor resistance was treated with ibrutinib combined with CAR-T cells, successfully bridged to allogeneic hematopoietic stem cell transplantation (allo-HSCT) , and the relative literatures were reviewed. Results: The patient was a young female with superficial lymph node enlarging at the beginning of the onset. Lymph node biopsy was confirmed as small lymphocytic lymphoma (SLL) without del (17p) . The disease progressed rapidly to CLL/SLL with del (17p) and bone marrow hematopoietic failure 2 years later. Firstly, the patient was treated with BCL-2 inhibitor (Venetoclax) , and the enlarged lymph nodes shrank significantly 2 months later. After 3 months, the disease progressed rapidly. The spleen was enlarged to 16 cm below the ribs, the neck lymph nodes was rapidly enlarged, and the superior vena cava syndrome appeared, which were mainly attributed to venetoclax resistance; so BTK inhibitor (ibrutinib) was used continuously after venetoclax discontinuation. Partial remission (PR) was achieved without lymphocytosis after 2 months, then ibrutinib was combined with CAR-T cells targeting CD19 antigen. Grade 1 of cytokine release syndrome (CRS) appeared after CAR-T cells infusion, and the complete remission (CR) was achieved after 1 month both in bone marrow and peripheral blood, with minimal residual disease (MRD) negative, then bridging allo-HSCT after 2 months of combined therapy. Conclusion: CLL/SLL patients with TP53 aberration have poor prognosis because of rapid progression, drug resistance, etc. Ibrutinib combined with CAR-T cell therapy can quickly achieved complete remission.
目的： 总结伊布替尼联合嵌合抗原受体T细胞（CAR-T）治疗TP53基因异常超高危慢性淋巴细胞白血病（CLL）/小细胞淋巴瘤（SLL）的经验，提高对此类疾病的认识。 方法： 报告1例伴del（17p）CLL/SLL患者BCL-2抑制剂耐药后，采用伊布替尼联合CAR-T成功治疗并顺利桥接异基因造血干细胞移植（allo-HSCT）的治疗经过，并进行文献复习。 结果： 患者为年轻女性，发病初表现为全身浅表淋巴结肿大，淋巴结活检病理确诊为SLL，无del（17p）；2年后疾病快速进展为CLL/SLL，伴del（17p），骨髓造血衰竭。采用BCL-2抑制剂维奈托克治疗2个月余肿大淋巴结明显缩小，3个月后疾病快速进展，脾脏增大至肋缘下16 cm，颈部淋巴结增大融合，并出现上腔静脉压迫综合征，考虑维奈托克耐药；遂更换为BTK抑制剂伊布替尼治疗，2个月余疾病达部分缓解（PR），联合靶向CD19的CAR-T治疗，细胞因子释放综合征（CRS）1级，1个月后达骨髓及外周血完全缓解（CR），且微小残留病（MRD）阴性，2个月后桥接allo-HSCT。 结论： TP53基因异常CLL/SLL临床进展快、易耐药、预后差，伊布替尼联合CAR-T治疗可快速达CR。.